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Pathogenesis, Diagnosis, and Management of Kikuchi–Fujimoto Disease Darcie Deaver, Phd, Pedro Horna, MD, Hernani Cualing, MD, and Lubomir Sokol, MD, Phd

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Pathogenesis, Diagnosis, and Management of Kikuchi–Fujimoto Disease Darcie Deaver, Phd, Pedro Horna, MD, Hernani Cualing, MD, and Lubomir Sokol, MD, Phd Kikuchi–Fujimoto disease is a rare lymphohistiocytic disorder that affects young women of Asian descent more frequently than persons of other ethnic groups. Net-Wing Beetle. Photograph courtesy of Sherri Damlo. www.damloedits.com. Pathogenesis, Diagnosis, and Management of Kikuchi–Fujimoto Disease Darcie Deaver, PhD, Pedro Horna, MD, Hernani Cualing, MD, and Lubomir Sokol, MD, PhD Background: Kikuchi–Fujimoto disease (KFD) is a rare lymphohistiocytic disorder with an unknown etiopathogenesis. This disease is misdiagnosed as malignant lymphoma in up to one-third of cases and is as- sociated with the development of systemic lupus erythematosus (SLE). Methods: The medical literature between the years 1972 and 2014 was searched for KFD, and the data were collected and analyzed regarding the epidemiology, clinical presentations, diagnosis, management, and suggested diagnostic and treatment algorithms. Results: Although KFD has been reported in other ethnic groups and geographical areas, it is more frequently diagnosed in young women of Asian descent. Patients with the disease typically present with rapidly evolving tender cervical lymphadenopathy, night sweats, fevers, and headache. Diagnosis is based on histopathological examination. Excisional lymph node biopsy is essential for a correct diagnosis. Apoptotic coagulation necrosis with karyorrhectic debris and the proliferation of histiocytes, plasmacytoid dendritic cells, and CD8+ T cells in the absence of neutrophils are characteristic cytomorphology features. Interface dermatitis at the onset of KFD may be a marker for the subsequent evolution of SLE. The natural course of the disease is typically benign. Short courses of steroids, nonsteroidal anti-inflammatory drugs, or hydroxychloroquine can be administered to patients with more severe symptoms. Conclusions: Although KFD was described more than 40 years ago, the etiology of this disease remains un- solved. Infectious or autoimmune processes were proposed but have not been definitively confirmed. Clinical presentation with systemic B symptoms and adenopathy may lead to an erroneous diagnosis of malignant lymphoma. The introduction of modern methods into hematopathology, including immunohistochemistry, flow cytometry, and molecular clonality studies, has decreased the probability of misdiagnosis. Until reliable prognostic markers are available, patients with KFD should have continued long-term follow-up care due to their increased risk of SLE. From the Departments of Malignant Hematology (DD, LS) and Address correspondence to Darcie Deaver, PhD, Celgene Hematopathology and Laboratory Medicine (PH) at the H. Lee Moffitt Corporation, 1503 Foppiano Loop, Round Rock, TX 78665. Cancer Center & Research Institute, Tampa, Florida, and IHCFLOW E-mail: [email protected] (HC), Lutz, Florida. No significant relationships exist between the authors and the com- Dr Deaver is now affiliated with Celgene Corporation. panies/organizations whose products or services may be referenced Submitted February 18, 2014; accepted July 2, 2014. in this article. October 2014, Vol. 21, No. 4 Cancer Control 313 Introduction and maculopapular rash. However, no confirmatory Kikuchi–Fujimoto disease (KFD), also known as studies or results from the control samples were avail- Kikuchi disease, is a rare lymphohistiocytic disor- able. Huh et al21 amplified sequences of HHV-8 from der first described in 1972.1,2 KFD generally affects lymph nodes in 6 out of 26 patients with KFD (23%). women of Asian descent between the ages of 20 and They did not detect any viral sequences in the reactive 35 years and has a male:female ratio of 1:2; howev- lymph nodes of the study controls, suggesting that er, new cases of KFD have also been described in HHV-8 may play a role in the pathogenesis of KFD. non-Asian ethnic groups and children in Europe and the United States.3-10 The acute or subacute onset of Parvovirus B19 adenopathy and systemic B symptoms in KFD has his- Zhang et al22 searched for parvovirus B19 in 33 lymph torically led to a misdiagnosis of malignant lymphoma, node samples from patients with KFD and 16 controls although modern hematopathological methods have using several different methods, including PCR, immu- made such misdiagnosis less likely.11-14 Following the nohistochemistry, and in situ hybridization (ISH). A resolution of KFD, concurrent autoimmune disorders significantly higher rate of B19 positivity was seen in have been reported; they may also be more frequently samples from patients with KFD than in controls; B19 diagnosed.15,16 infected cells were mostly composed of lymphocytes and a small number of histiocytes.22 Epidemiology The precise incidence of KFD is unknown; however, Epstein–Barr Virus a large review identified and analyzed 733 patients Hudnall et al23 tested 30 lymph node samples of pa- diagnosed worldwide since 1972.8 Of those cases, 140 tients with KFD and 12 controls for the presence (19%) were pediatric patients, and the male:female of EBV using real-time PCR, EBV-encoded RNA ISH, ratio was 1.4:1.8 (It is worth noting that a higher and EBV latent membrane protein with immunohis- propensity for male sex has only been observed in tochemistry. Cells with apoptotic features positive for children younger than 12 years of age.5) The pre- EBV-encoded RNA were found in the necrotic regions senting symptoms in children are similar to adults, of many KFD cases, suggesting that the disease could although fever and rash are more frequent in pediatric be due to a hyperimmune reaction against EBV infec- patients.8 Children younger than 18 years of age may tion. Hollingsworth et al24 looked for EBV and HHV-6 also demonstrate bilateral cervical lymphadenopathy using PCR and ISH in 20 patients with KFD, but the more frequently than adults.9,10 study results did not support a viral role in the patho- The occurrence of KFD in family members has genesis of KFD. However, Yen et al25 reported EBV rarely been described.17 A search for a link between infection in a child with a cutaneous manifestation of KFD and human leukocyte antigen class 2 alleles KFD, supporting the pathogenic role of EBV infection, in the Japanese population suggested the possibil- and Chiu et al26 detected EBV RNA sequences in all ity of a positive relationship between DPA1*01 and 10 tested samples of KFD but found only a single DPB1*0202 alleles and the disease.18 Because these al- case of EBV-encoded proteins. No evidence of human leles are much more frequent in Japan than in Europe T-lymphotrophic virus or parvovirus B19 was seen in and the United States, this finding may explain the the patient samples of this study.26 higher prevalence of KFD in patients of Asian descent, Overall, the results of studies searching for a viral thus supporting a possible autoimmune pathogenic etiology of KFD have been inconsistent. Laboratory mechanism. methods differed among laboratories, positive results were documented in a small number of samples, and Viral Infections confirmatory studies with control samples were often Epstein–Barr virus (EBV), human herpesvirus (HHV) unavailable. Currently, no definitive evidence suggests types 6, 7, and 8, herpes simplex virus, HIV, human that a known virus plays a key role in the pathogen- T-lymphotrophic virus, and parvovirus B19 are the esis of KFD. most frequently studied viruses in patients with KFD. Autoimmune Mechanism Human Herpesviruses Autoimmune disorders are frequently reported in Cho et al19 studied HHV-6 and HHV-7 in 50 archival patients with KFD, with systemic lupus erythema- samples of KFD and 20 controls using nested poly- tosus (SLE) being the most common disorder linked merase chain reaction (PCR) and found no significant to KFD.27 In many reports, KFD preceded the de- difference in the viral DNA sequences between patients velopment of SLE; however, the diagnosis of KFD and controls. Labrador et al20 identified the DNA of has been reported to simultaneously occur or follow HHV-7 in the affected lymph node of a young patient the diagnosis of SLE.27,28 In one study, patients with with KFD who presented with cervical adenopathy KFD were negative for antinuclear and anti-DNA an- 314 Cancer Control October 2014, Vol. 21, No. 4 tibodies, suggesting that — at least initially — SLE SLE adenopathy is usually mild, generalized, and KFD have distinct pathogenic mechanisms.4 and nontender. The cytomorphology of enlarged A diagnosis of SLE requires at least 4 out of 11 criteria lymph nodes in SLE consists of scattered plasma to be present, and lymphadenitis is not included in cells and immunoblasts, increased vascularity with these criteria.29 Sopeña et al30 found several autoim- Azzopardi phenomenon associated with moderate mune disorders, including SLE, Sjögren syndrome, reactive follicular hyperplasia, or varying degrees of thyroiditis, and leukocytoclastic vasculitis, in 9 (53%) coagulative necrosis with the presence of hematox- of 20 patients with KFD. ylin bodies (Table 1).4,10,27 These features can help Kim et al31 reviewed patients with KFD in the differentiate involvement with SLE compared with context of SLE and noticed an increasing number KFD.27,34 SLE is also associated with higher antinu- of case reports in the medical literature. Among clear antibody titers and organomegaly, which is a 9 cases of KFD and SLE, 7 patients manifested with rare finding in KFD.35 skin disease. Histological evaluation of skin biopsies was consistent with SLE in 3 of the 7 cases. Com- Molecular Biology monly, patients with a simultaneous onset of KFD Molecular pathways implicated in the pathobi- and SLE have flare-ups of lupus; therefore, some re- ology of KFD are not well understood. Ishimura searchers have suggested that concurrent KFD and et al36 reported on a noninvasive method for di- SLE diagnoses are actually lupus lymphadenitis.31 agnosing KFD using gene expression profiling Other researchers suggest that KFD is a forme fruste on peripheral mononuclear cells. The top 5 up- of SLE.32 Lymphadenitis is not included among the regulated genes included FI44L, CXCL10, GBP1, 11 diagnostic criteria of SLE, so it cannot establish EPSTI1, and IFI27.
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